cystic fibrosis
Cystic fibrosis (CF) is an inherited disease that causes the body to produce mucus that's extremely thick and sticky. The mucus in people with CF is thicker than normal because CF affects cells in the epithelium (pronounced: eh-puh-thee-lee-um), the layer of cells that lines the passages in the body's organs. In a person who does not have CF, the epithelial cells produce a thin, watery mucus that acts like a lubricant and helps protect the body's tissues. In a person with CF, however, the thicker mucus doesn't move as easily. This thick, sticky mucus clogs passages in many of the body's organs and infection sets in.
The two organs that are most affected are the lungs and pancreas, where the thick mucus causes breathing and digestive problems. The thicker mucus has trouble moving out of the lungs, so bacteria can remain and cause infections. The thick mucus can also be found in the pancreas — an organ that produces proteins called enzymes that flow into the intestine to support the body's digestion process. Because the mucus can block the path between the pancreas and the intestines, people with CF have trouble digesting food and getting the vitamins and nutrients they need from it.
This condition is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition.
Mutations in the CFTR gene cause cystic fibrosis.
The CFTR gene provides instructions for making a channel that transports negatively charged particles called chloride ions into and out of cells. The flow of chloride ions helps control the movement of water in tissues, which is necessary for the production of thin, freely flowing mucus.
Mutations in the CFTR gene disrupt the function of the chloride channels, preventing them from regulating the flow of chloride ions and water across cell membranes. As a result, cells that line the passageways of the lungs, pancreas, and other organs produce mucus that is unusually thick and sticky. This mucus clogs the airways and glands, causing the characteristic signs and symptoms of cystic fibrosis.
Other genetic and environmental factors likely influence the severity of the condition. For example, mutations in genes other than CFTR might help explain why some people with cystic fibrosis are more severely affected than others. Most of these genetic changes have not been identified, however.
Cystic fibrosis signs and symptoms can vary from child to child, depending on the severity of the disease. Even in the same child, symptoms may worsen or improve as time passes. In some children, symptoms begin during infancy. Other people may not begin experiencing symptoms until adolescence or adulthood.
One of the first signs of cystic fibrosis is an excessively salty taste to the skin. People with cystic fibrosis tend to have higher than normal amounts of salt in their sweat. Parents often can taste the salt when they kiss their child. Most of the other signs and symptoms of cystic fibrosis affect the respiratory system or the digestive system. Respiratory signs and symptoms
The thick and sticky mucus associated with cystic fibrosis clogs the tubes that carry air in and out of your lungs. This can cause:
Persistent cough
Wheezing
Repeated lung infections
Repeated sinus infections
Digestive signs and symptoms
The thick mucus can also block tubes that carry digestive enzymes from your pancreas to your small intestine. Without these digestive enzymes, your intestines can't fully absorb the nutrients in the food you eat. This can cause:
Foul-smelling, greasy stools
Poor weight gain and growth
Distended abdomen from constipation
Intestinal blockage, particularly in newborns
At present, neither gene therapy nor any other kind of treatment exists for the basic causes of Cystic Fibrosis, although several drug-based approaches are being investigated. In the meantime, the best that doctors can do is to ease the symptoms of Cystic Fibrosis or slow the progress of the disease so the patient's quality of life is improved. This is achieved by antibiotic therapy combined with treatments to clear the thick mucus from the lungs. The therapy is tailored to the needs of each patient. For patients whose disease is very advanced, lung transplantation may be an option. Cystic Fibrosis was once always fatal in childhood. Better treatment methods developed over the past 20 years have increased the average lifespan of Cystic Fibrosis patients to nearly 30 years. These treatment approaches are detailed more fully below:
Prognosis of Cystic Fibrosis: The predicted median age of survival for a person with CF is 37 years. However, with the continuous introduction of many new treatments, the life expectancy of a person with CF is increasing to ages as high as 40 or 50. Lung transplantation is often necessary as CF worsens .
incidence of cystic fibrosis:2,500 babies annually USA; 1 in 3,000 Caucasian babies.
Cystic fibrosis is the most common lethal disease inherited by the white population. Cystic fibrosis is inherited as an autosomal recessive trait. In the United States white population of Northern European origin, prevalence is 1 case per 3,200 population. In blacks, prevalence is 1 case per 15,000 population. In Hispanics, prevalence is 1 case per 9,200 population. In Asian Americans, prevalence is 1 case per 31,000 population.
discussion questions :
1.What is cystic fibrosis?
2.What people have cystic fibrosis?
3.How many people have cystic fibrosis?
work cited:
cystic fibrosis
Cystic fibrosis (CF) is an inherited disease that causes the body to produce mucus that's extremely thick and sticky. The mucus in people with CF is thicker than normal because CF affects cells in the epithelium (pronounced: eh-puh-thee-lee-um), the layer of cells that lines the passages in the body's organs. In a person who does not have CF, the epithelial cells produce a thin, watery mucus that acts like a lubricant and helps protect the body's tissues. In a person with CF, however, the thicker mucus doesn't move as easily. This thick, sticky mucus clogs passages in many of the body's organs and infection sets in.
The two organs that are most affected are the lungs and pancreas, where the thick mucus causes breathing and digestive problems. The thicker mucus has trouble moving out of the lungs, so bacteria can remain and cause infections. The thick mucus can also be found in the pancreas — an organ that produces proteins called enzymes that flow into the intestine to support the body's digestion process. Because the mucus can block the path between the pancreas and the intestines, people with CF have trouble digesting food and getting the vitamins and nutrients they need from it.
This condition is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition.
Mutations in the CFTR gene cause cystic fibrosis.
The CFTR gene provides instructions for making a channel that transports negatively charged particles called chloride ions into and out of cells. The flow of chloride ions helps control the movement of water in tissues, which is necessary for the production of thin, freely flowing mucus.
Mutations in the CFTR gene disrupt the function of the chloride channels, preventing them from regulating the flow of chloride ions and water across cell membranes. As a result, cells that line the passageways of the lungs, pancreas, and other organs produce mucus that is unusually thick and sticky. This mucus clogs the airways and glands, causing the characteristic signs and symptoms of cystic fibrosis.
Other genetic and environmental factors likely influence the severity of the condition. For example, mutations in genes other than CFTR might help explain why some people with cystic fibrosis are more severely affected than others. Most of these genetic changes have not been identified, however.
Cystic fibrosis signs and symptoms can vary from child to child, depending on the severity of the disease. Even in the same child, symptoms may worsen or improve as time passes. In some children, symptoms begin during infancy. Other people may not begin experiencing symptoms until adolescence or adulthood. One of the first signs of cystic fibrosis is an excessively salty taste to the skin. People with cystic fibrosis tend to have higher than normal amounts of salt in their sweat. Parents often can taste the salt when they kiss their child. Most of the other signs and symptoms of cystic fibrosis affect the respiratory system or the digestive system. Respiratory signs and symptoms
The thick and sticky mucus associated with cystic fibrosis clogs the tubes that carry air in and out of your lungs. This can cause:
Persistent cough
Wheezing
Repeated lung infections
Repeated sinus infections
Digestive signs and symptoms
The thick mucus can also block tubes that carry digestive enzymes from your pancreas to your small intestine. Without these digestive enzymes, your intestines can't fully absorb the nutrients in the food you eat. This can cause:
Foul-smelling, greasy stools
Poor weight gain and growth
Distended abdomen from constipation
Intestinal blockage, particularly in newborns
At present, neither gene therapy nor any other kind of treatment exists for the basic causes of Cystic Fibrosis, although several drug-based approaches are being investigated. In the meantime, the best that doctors can do is to ease the symptoms of Cystic Fibrosis or slow the progress of the disease so the patient's quality of life is improved. This is achieved by antibiotic therapy combined with treatments to clear the thick mucus from the lungs. The therapy is tailored to the needs of each patient. For patients whose disease is very advanced, lung transplantation may be an option. Cystic Fibrosis was once always fatal in childhood. Better treatment methods developed over the past 20 years have increased the average lifespan of Cystic Fibrosis patients to nearly 30 years. These treatment approaches are detailed more fully below:
Prognosis of Cystic Fibrosis: The predicted median age of survival for a person with CF is 37 years. However, with the continuous introduction of many new treatments, the life expectancy of a person with CF is increasing to ages as high as 40 or 50. Lung transplantation is often necessary as CF worsens .
incidence of cystic fibrosis:2,500 babies annually USA; 1 in 3,000 Caucasian babies.
Cystic fibrosis is the most common lethal disease inherited by the white population. Cystic fibrosis is inherited as an autosomal recessive trait. In the United States white population of Northern European origin, prevalence is 1 case per 3,200 population. In blacks, prevalence is 1 case per 15,000 population. In Hispanics, prevalence is 1 case per 9,200 population. In Asian Americans, prevalence is 1 case per 31,000 population.
cystic fibrosis
Cystic fibrosis (CF) is an inherited disease that causes the body to produce mucus that's extremely thick and sticky. The mucus in people with CF is thicker than normal because CF affects cells in the epithelium (pronounced: eh-puh-thee-lee-um), the layer of cells that lines the passages in the body's organs. In a person who does not have CF, the epithelial cells produce a thin, watery mucus that acts like a lubricant and helps protect the body's tissues. In a person with CF, however, the thicker mucus doesn't move as easily. This thick, sticky mucus clogs passages in many of the body's organs and infection sets in.
The two organs that are most affected are the lungs and pancreas, where the thick mucus causes breathing and digestive problems. The thicker mucus has trouble moving out of the lungs, so bacteria can remain and cause infections. The thick mucus can also be found in the pancreas — an organ that produces proteins called enzymes that flow into the intestine to support the body's digestion process. Because the mucus can block the path between the pancreas and the intestines, people with CF have trouble digesting food and getting the vitamins and nutrients they need from it.
This condition is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition.
Mutations in the CFTR gene cause cystic fibrosis.
The CFTR gene provides instructions for making a channel that transports negatively charged particles called chloride ions into and out of cells. The flow of chloride ions helps control the movement of water in tissues, which is necessary for the production of thin, freely flowing mucus.
Mutations in the CFTR gene disrupt the function of the chloride channels, preventing them from regulating the flow of chloride ions and water across cell membranes. As a result, cells that line the passageways of the lungs, pancreas, and other organs produce mucus that is unusually thick and sticky. This mucus clogs the airways and glands, causing the characteristic signs and symptoms of cystic fibrosis.
Other genetic and environmental factors likely influence the severity of the condition. For example, mutations in genes other than CFTR might help explain why some people with cystic fibrosis are more severely affected than others. Most of these genetic changes have not been identified, however.
Cystic fibrosis signs and symptoms can vary from child to child, depending on the severity of the disease. Even in the same child, symptoms may worsen or improve as time passes. In some children, symptoms begin during infancy. Other people may not begin experiencing symptoms until adolescence or adulthood.
One of the first signs of cystic fibrosis is an excessively salty taste to the skin. People with cystic fibrosis tend to have higher than normal amounts of salt in their sweat. Parents often can taste the salt when they kiss their child.
Most of the other signs and symptoms of cystic fibrosis affect the respiratory system or the digestive system.
Respiratory signs and symptoms
The thick and sticky mucus associated with cystic fibrosis clogs the tubes that carry air in and out of your lungs. This can cause:
- Persistent cough
- Wheezing
- Repeated lung infections
- Repeated sinus infections
Digestive signs and symptomsThe thick mucus can also block tubes that carry digestive enzymes from your pancreas to your small intestine. Without these digestive enzymes, your intestines can't fully absorb the nutrients in the food you eat. This can cause:
At present, neither gene therapy nor any other kind of treatment exists for the basic causes of Cystic Fibrosis, although several drug-based approaches are being investigated. In the meantime, the best that doctors can do is to ease the symptoms of Cystic Fibrosis or slow the progress of the disease so the patient's quality of life is improved. This is achieved by antibiotic therapy combined with treatments to clear the thick mucus from the lungs. The therapy is tailored to the needs of each patient. For patients whose disease is very advanced, lung transplantation may be an option.
Cystic Fibrosis was once always fatal in childhood. Better treatment methods developed over the past 20 years have increased the average lifespan of Cystic Fibrosis patients to nearly 30 years. These treatment approaches are detailed more fully below:
Prognosis of Cystic Fibrosis: The predicted median age of survival for a person with CF is 37 years. However, with the continuous introduction of many new treatments, the life expectancy of a person with CF is increasing to ages as high as 40 or 50. Lung transplantation is often necessary as CF worsens .
incidence of cystic fibrosis:2,500 babies annually USA; 1 in 3,000 Caucasian babies.
Cystic fibrosis is the most common lethal disease inherited by the white population. Cystic fibrosis is inherited as an autosomal recessive trait. In the United States white population of Northern European origin, prevalence is 1 case per 3,200 population. In blacks, prevalence is 1 case per 15,000 population. In Hispanics, prevalence is 1 case per 9,200 population. In Asian Americans, prevalence is 1 case per 31,000 population.
discussion questions :
1.What is cystic fibrosis?
2.What people have cystic fibrosis?
3.How many people have cystic fibrosis?
work cited:
cystic fibrosis
Cystic fibrosis (CF) is an inherited disease that causes the body to produce mucus that's extremely thick and sticky. The mucus in people with CF is thicker than normal because CF affects cells in the epithelium (pronounced: eh-puh-thee-lee-um), the layer of cells that lines the passages in the body's organs. In a person who does not have CF, the epithelial cells produce a thin, watery mucus that acts like a lubricant and helps protect the body's tissues. In a person with CF, however, the thicker mucus doesn't move as easily. This thick, sticky mucus clogs passages in many of the body's organs and infection sets in.
The two organs that are most affected are the lungs and pancreas, where the thick mucus causes breathing and digestive problems. The thicker mucus has trouble moving out of the lungs, so bacteria can remain and cause infections. The thick mucus can also be found in the pancreas — an organ that produces proteins called enzymes that flow into the intestine to support the body's digestion process. Because the mucus can block the path between the pancreas and the intestines, people with CF have trouble digesting food and getting the vitamins and nutrients they need from it.
This condition is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition.
Mutations in the CFTR gene cause cystic fibrosis.
The CFTR gene provides instructions for making a channel that transports negatively charged particles called chloride ions into and out of cells. The flow of chloride ions helps control the movement of water in tissues, which is necessary for the production of thin, freely flowing mucus.
Mutations in the CFTR gene disrupt the function of the chloride channels, preventing them from regulating the flow of chloride ions and water across cell membranes. As a result, cells that line the passageways of the lungs, pancreas, and other organs produce mucus that is unusually thick and sticky. This mucus clogs the airways and glands, causing the characteristic signs and symptoms of cystic fibrosis.
Other genetic and environmental factors likely influence the severity of the condition. For example, mutations in genes other than CFTR might help explain why some people with cystic fibrosis are more severely affected than others. Most of these genetic changes have not been identified, however.
Cystic fibrosis signs and symptoms can vary from child to child, depending on the severity of the disease. Even in the same child, symptoms may worsen or improve as time passes. In some children, symptoms begin during infancy. Other people may not begin experiencing symptoms until adolescence or adulthood.
One of the first signs of cystic fibrosis is an excessively salty taste to the skin. People with cystic fibrosis tend to have higher than normal amounts of salt in their sweat. Parents often can taste the salt when they kiss their child.
Most of the other signs and symptoms of cystic fibrosis affect the respiratory system or the digestive system.
Respiratory signs and symptoms
The thick and sticky mucus associated with cystic fibrosis clogs the tubes that carry air in and out of your lungs. This can cause:
- Persistent cough
- Wheezing
- Repeated lung infections
- Repeated sinus infections
Digestive signs and symptomsThe thick mucus can also block tubes that carry digestive enzymes from your pancreas to your small intestine. Without these digestive enzymes, your intestines can't fully absorb the nutrients in the food you eat. This can cause:
At present, neither gene therapy nor any other kind of treatment exists for the basic causes of Cystic Fibrosis, although several drug-based approaches are being investigated. In the meantime, the best that doctors can do is to ease the symptoms of Cystic Fibrosis or slow the progress of the disease so the patient's quality of life is improved. This is achieved by antibiotic therapy combined with treatments to clear the thick mucus from the lungs. The therapy is tailored to the needs of each patient. For patients whose disease is very advanced, lung transplantation may be an option.
Cystic Fibrosis was once always fatal in childhood. Better treatment methods developed over the past 20 years have increased the average lifespan of Cystic Fibrosis patients to nearly 30 years. These treatment approaches are detailed more fully below:
Prognosis of Cystic Fibrosis: The predicted median age of survival for a person with CF is 37 years. However, with the continuous introduction of many new treatments, the life expectancy of a person with CF is increasing to ages as high as 40 or 50. Lung transplantation is often necessary as CF worsens .
incidence of cystic fibrosis:2,500 babies annually USA; 1 in 3,000 Caucasian babies.
Cystic fibrosis is the most common lethal disease inherited by the white population. Cystic fibrosis is inherited as an autosomal recessive trait. In the United States white population of Northern European origin, prevalence is 1 case per 3,200 population. In blacks, prevalence is 1 case per 15,000 population. In Hispanics, prevalence is 1 case per 9,200 population. In Asian Americans, prevalence is 1 case per 31,000 population.
discussion questions :
1.What is cystic fibrosis?
2.What people have cystic fibrosis?
3.How many people have cystic fibrosis?
Works Cited: